Program Operations Manual System (POMS)
TN 31 (08-20)
DI 23022.927 Alveolar Soft Part Sarcoma
COMPASSIONATE ALLOWANCES INFORMATION
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ALVEOLAR SOFT
PART SARCOMA |
ALTERNATE NAMES
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ASP Sarcoma; ASPS
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DESCRIPTION
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Alveolar Soft Part Sarcoma (ASP) is a rare type of soft tissue sarcoma occurring primarily in children and adolescents.
These tumors are usually located in the head and neck, arms or legs (usually the deep
thigh area), chest, abdomen, genital organs, or anal area.
These tumors usually arise in the soft tissues containing many blood vessels, but
can also grow inside the bones. The exact cause of ASP is unknown, but some scientific
research suggests that genetic mutations in the ASPL and TFE3 genes are contributory
to the cause of ASP.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-
CM
CODING
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Diagnostic testing:
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Bone marrow aspiration and biopsy;
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Immunohistochemistry study; and
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Physical findings: Physical findings of ASP depend on the site of the tumor and degree of metastases.
These signs may include:
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A painless swelling or lump;
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Pain or soreness caused by compressed nerves or muscles; and
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Limping or other difficulty using the legs and feet.
ICD-9: 171.9
ICD-10: C49
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PROGRESSION
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ASP occurs mainly in children between the ages of infancy to approximately 19 years
of age. The prognosis is poor if there are metastases to other sites. Persons with
ASP tumors may relapse several years after a prolonged period of apparent remission.
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TREATMENT
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Treatment of ASP tumors depends on the specific type of tumor, its size, its location,
and the amount that it has spread (metastasis). The standard treatment of these tumors
is a complete resection of the primary tumor. If complete excision is not feasible,
radiation therapy is administered. Adjuvant chemotherapy is used in some patients
with varying response to treatment.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets
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13.04
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Listing level severity of soft tissue sarcomas in adults must be documented by metastases,
or by recurrence or persistence following initial anti-neoplastic treatment.
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113.03
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Listing level severity in children must be documented by confirmed diagnosis or recurrence. |
Equals
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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